Learn More - Dent Disease
What is Dent Disease?
Dent Disease is a rare genetic disorder that affects the kidneys. Typical problems are leakage of proteins into the urine and large amounts of calcium in the urine. Kidney stones are common. People with Dent Disease can develop kidney damage, even kidney failure. Kidney dialysis or transplantation may be necessary. The disease is maybe diagnosed in childhood, but can be silent for years.
Who gets Dent Disease?
Males are more often affected by Dent disease than women, but women can also have a mild case of the disease. Dent affects people all over the world. Currently we do not know how many people have Dent.
What causes Dent Disease?
The most common Dent disease is a defect in two genes. There are two main types of Dent, type 1 and type2. Type 1 is more common, which is caused by a defect in a protein gene can cause proteinuria (increased amounts of protein in urine). Type 2 is an enzyme abnormality in kidney cell function called that causes hypercalciuria (large amounts of calcium in the urine).
How is Dent Disease diagnosed?
Patients that have kidney stone with proteinuria and hypercalciuria can have a genetic test to confirm which gene is not functioning correctly.
What is/is there treatment for Dent Disease?
There is currently no cure for Dent disease, but there are treatments we can offer. Some medications (diuretics) can be given to lower the calcium in the urine and hopefully reduce the risk of kidney stones. Studies have shown that a high citrate diet may delay the loss of kidney function, so potassium citrate can be given to patients with Dent Disease. However, studies in patients are badly needed to determine if either of these treatments is effective in Dent Disease.
